What is Guillain-Barr syndrome?

104

Guillain-Barre syndrome, or GBS, is a disorder in which the

bodys immune system attacks the peripheral nervous system.
Guillain-Barr (ghee-yan bah-ray) syndrome, or GBS, is a disorder in which the bodys
immune system attacks part of the peripheral nervous system. The peripheral nervous
system includes the cranial nerves (except the optic [eye] nerve), the spinal nerves, and
the autonomic nervous system that governs involuntary actions. The central nervous
system, which is composed of the spinal cord and brain, is not directly involved by
Guillain-Barr syndrome, as opposed to certain other immune-mediated neurologic
diseases such as multiple sclerosis.
Guillain-Barr syndrome includes several variants, the most common of which is a
multifocal demyelinating disorder of the peripheral nerves referred to as an acute
inflammatory demyelinating polyneuropathy (AIDP). Some cases of Guillain-Barr
syndrome are associated with a primarily motor axonal process (acute motor axonal
neuropathy; AMAN) with axonal degeneration (axons are long, thin extensions of the
nerve cells and carry nerve signals) and sparing of the myelin (the myelin is an
electrically insulating phospholipid layer that surrounds the axons of many neurons and
is not spared by AIDP). Other cases appear to involve both sensory and motor axons
and such cases are termed acute motor and sensory axonal neuropathy (AMSAN).
More than 90 percent of patients with Guillain-Barr syndrome in Europe and North
America have AIDP. AMAN occurs in less than 10 percent of persons with Guillain-Barr
syndrome in the western hemisphere but in more than 40 percent of those affected in
China and Japan. The incidence of AMSAN is very low (less than 10 percent of that of
AMAN).
Miller Fisher syndrome (MFS) is another Guillain-Barr syndrome variant that occurs in
about 5 percent of people affected by Guillain-Barr syndrome. It is characterized by
ophthalmoplegia (eye muscle weakness), areflexia (absence of reflexes), ataxia (the
inability to coordinate voluntary muscular movements such as walking), and, in some
cases, facial and bulbar palsy (affecting vital functions regulated by the brain stem, like
breathing, and swallowing or speech).
Guillain-Barr syndrome can strike at any age but there are peaks in young adults and
the elderly; men may be more likely to develop Guillain-Barr syndrome than women.
Seasonality has not been reported in developed countries like the United States [1].
Although this syndrome is rare (affecting about one to two persons in 100,000), it is the
most common cause of acute neuromuscular paralysis in the world.

Where does Guillain-Barr syndrome come from?

Guillain-Barr syndrome often occurs a few days or weeks after a person has had
symptoms of a respiratory or gastrointestinal viral or bacterial infection; in fact, twothirds of affected individuals have had a preceding infection. Campylobacter jejuni, a
common bacterial cause of gastrointestinal infection, is the most common infection
associated with Guillain-Barr syndrome and may precede over 25 percent of GuillainBarr syndrome cases. Less commonly, other infections such as cytomegalovirus,
Epstein-Barr virus, and Mycoplasma pneumonia may precede this syndrome. Rarely,
surgery, trauma, bone marrow transplantation, certain systemic illnesses, and the use of
some medications or vaccinations (e.g. influenza vaccination) will trigger Guillain-Barr
syndrome.
Despite the observation that a large percentage of Guillain-Barr syndrome cases follow
infection, Guillain-Barr syndrome itself is not contagious.
No one yet knows why Guillain-Barr syndrome strikes some people and not others. Nor
does anyone know exactly what sets the disease in motion. What scientists do know is
that the bodys immune system begins to attack the body itself, causing what is known
as an autoimmune disease. Usually the cells of the immune system attack only foreign
material and invading organisms but in the case of Guillain-Barr syndrome, the bodys
response to foreign material and infection results in an immune response that crossreacts with components of the hosts own peripheral nervous system. This is thought to
occur because the hosts peripheral nervous system may share certain similarities with
the foreign material or invading organisms at the molecular level. This is known as
molecular mimicry.
In Guillain-Barr syndrome, the immune system starts to destroy the myelin sheath that
surrounds the axons of many peripheral nerves, or even the axons themselves. The
myelin sheath surrounding the axon speeds up the transmission of nerve signals and
allows the transmission of signals over long distances. In diseases in which these myelin
sheaths are injured or degraded, the nerves cannot transmit signals efficiently. That is
why the muscles begin to lose their ability to respond to commands from the brain that
must be carried through the nerve network. The brain also receives fewer sensory
signals from the rest of the body, resulting in an inability to feel textures, heat, pain, and
other sensations. Alternately, the brain may receive inappropriate signals that result in
tingling, crawling-skin, or painful sensations known as paresthesias. Because the
signals to and from the arms and legs must travel the longest distances, they are most
vulnerable to interruption. Therefore, muscle weakness and tingling sensations usually
first appear in the hands and feet and progress upwards.

Symptoms of Guillain-Barre syndrome

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GBS initially causes weakness or tingling sensations in the

legs. These symptoms can progress up the body and in severity,
and can lead to paralysis.
The first symptoms of Guillain-Barr syndrome include varying degrees of weakness or
tingling sensations in the legs. The weakness and abnormal sensations may spread to
the arms and upper body. These symptoms can increase in intensity until certain
muscles cannot be used at all and, when severe, the affected person is almost totally
paralyzed. In these cases the disorder is life threateningpotentially interfering with
breathing and, at times, with blood pressure or heart rateand is considered a medical
emergency. Bladder dysfunction and constipation may occur. Fever is not present at the
initial presentation of Guillain-Barr syndrome.
After the first clinical manifestations of Guillain-Barr syndrome, the symptoms can
progress over the course of hours, days, or weeks. Most people reach the stage of
greatest weakness within the first two weeks after symptoms appear, and by the third
week of the illness 90 percent of affected individuals are at their weakest.
Persons with Guillain-Barr syndrome are often hospitalized and closely monitored while
diagnostic tests are performed. This is because 10-30 percent may eventually require
mechanical ventilation and intensive care due to interference with breathing. Although
both sides of the body are reported to have symmetrical involvement, a lack of
symmetry of clinical and electrophysiological findings has been reported. [2]
In contrast to the relatively uniform speed of recovery among those with acute
inflammatory demyelinating polyneuropathy (AIDP), two patterns of recovery are found
with acute motor axonal neuropathy; AMAN. Some individuals recover quickly within
days and others experience slow and poor recovery. Acute motor and sensory axonal
neuropathy (AMSAN) tends to be associated with severe illness and slow recovery.

What are the serious and long-term risks of Guillain-Barr

syndrome?
Guillain-Barr syndrome can be a devastating disorder because of its sudden and
unexpected onset. In addition, recovery is not necessarily quick. Affected persons
usually reach the point of greatest weakness or paralysis days or weeks after the first
symptoms occur. Symptoms then stabilize at this level for a period of days, weeks, or,
sometimes, months. The recovery period may be as little as a few weeks or as long as a
few years. About 30 percent of those with Guillain-Barr syndrome still have residual
weakness after three years. About 3 percent may suffer a relapse of muscle weakness
and tingling sensations many years after the initial attack.

Those affected by Guillain-Barr syndrome face not only physical difficulties, but
emotionally painful periods as well. It is often extremely difficult for patients to adjust to
sudden paralysis and dependence on others for help with routine daily activities. Patients
sometimes need psychological counseling to help them adapt.

How is Guillain-Barre syndrome

(GBS) diagnosed?
54

Guillain-Barr syndrome is characterized by rapid paralysis on

both sides of the body. Other unique symptoms distinguish it
from other syndromes.
Guillain-Barr syndrome is called a syndrome rather than a disease because it is not
clear that a single specific disease-causing agent is involved. A syndrome is a medical
condition characterized by a constellation of certain symptoms (what the affected person
feels) and signs (what a health care provider can observe or measure). The signs and
symptoms of Guillain-Barr syndrome can be quite varied, so, on rare occasions, it may
be difficult to diagnose in its earliest stages.
Several disorders have symptoms similar to those found in Guillain-Barr syndrome.
Collectively, the signs and symptoms form a certain pattern that helps to differentiate
Guillain-Barr syndrome from other disorders. For example, if the symptoms appear on
both sides of the body (most common in Guillain-Barr syndrome) and symptoms
appear rapidly (in other disorders, muscle weakness may progress over months rather
than days or weeks), it is more likely to be Guillain-Barr syndrome. In addition,
reflexes such as knee jerks are usually lost. Because the signals traveling along the
nerve are slower, a nerve conduction velocity test can give clues to aid in the diagnosis.
The cerebrospinal fluid (CSF) that bathes the spinal cord and brain contains more protein
than usual in Guillain-Barr syndrome [3] but has a normal CSF cell count. Therefore, a
spinal tap (a procedure in which the doctor inserts a needle into the patients lower back
to draw cerebrospinal fluid from the spinal column) may need to be performed. The
peripheral white blood cell count in Guillain-Barr syndrome is normal.
The two most common forms can be differentiated with specific tests. In the acute
inflammatory demyelinating polyneuropathy (AIDP) form, immune system reactions
result in demyelination. It is diagnosed when nerve conduction studies show slowing of
nerve conduction suggestive of demyelination in two or more motor nerves. Acute
motor axonal neuropathy (AMAN) is diagnosed when nerve conduction studies show a
reduction of compound muscle action potential without significant conduction slowing.
Testing for serum antibodies to certain nerve components may be useful for confirming
the diagnosis of Miller Fisher syndrome (MFS).

Treatment for Guillain-Barre

syndrome (GBS)
14

The most critical part of treatment for GBS involves keeping a

persons body functioning during recovery of the nervous
sytem.
There is no known cure for Guillain-Barr syndrome (GBS). The most critical part of the
treatment for this syndrome consists of supportive care directed at keeping the persons
body functioning during recovery of the nervous system. This can sometimes require
placing the affected person on a respirator, a heart monitor, or other machines that
assist body function. The need for this sophisticated machinery is one reason why people
are usually treated in hospitals, often in an intensive care ward.
In the hospital, doctors can also look for and treat the many problems that can afflict
any paralyzed person - complications such as pneumonia, pressure ulcers (bed sores)
and deep venous thrombosis (blood clots). Often, even before recovery begins,
caregivers may be instructed to manually move the affected persons limbs to help keep
the muscles flexible to avoid contractures and to keep muscles strong. Later, as the
individual begins to recover limb control, physical therapy begins.
Disease-modifying therapies are available that significantly lessen the severity of the
illness and accelerate the recovery in most people. Currently, plasma exchange (also
known as plasmapheresis) and high-dose immunoglobulin therapy are used. Both of
them are equally effective, but immunoglobulin is easier to administer. The combination
of both therapies is not superior to either method. Plasma exchange is a method by
which whole blood is removed from the body and processed so that the red and white
blood cells are separated from the plasma, or liquid portion of the blood. The blood cells
are then returned to the patient without the plasma, which the body quickly replaces.
Scientists still do not understand exactly how plasma exchange works, but the technique
seems to reduce the severity and duration of the Guillain-Barr syndrome episode. This
may be because the plasma portion of the blood contains elements of the immune
system that may be toxic to myelin.
In high-dose immunoglobulin therapy, doctors give intravenous injections of the proteins
that, in small quantities, the immune system uses naturally to attack invading
organisms. Immunoglobulin is derived from a pool of thousands of normal donors.
Investigators have found that giving high doses of immunoglobulin to Guillain-Barr
syndrome patients can lessen the immune attack on the nervous system but the precise
mechanism of action is unknown.
The use of steroid hormones has also been tried as a way to reduce the severity of
Guillain-Barr syndrome, but controlled clinical trials have demonstrated that this
treatment not only ineffective but may even have a harmful effect. Carefully planned

clinical trials of new and experimental therapies are the key to improving the treatment
of persons with Guillain-Barr syndrome.

Preventing Guillain-Barre syndrome

(GBS)
52

Doctors and scientists have not yet determined how to prevent

Guinnain-Barr syndrome.
Since Guillain-Barr syndrome is not a disease itself, and it is not known exactly how it
occurs, it is difficult to say how Guillain-Barr Syndrome could be prevented. Scientists
are concentrating on finding new treatments and refining existing ones. Scientists are
also looking at the workings of the immune system to find which cells are responsible for
beginning and carrying out the attack on the nervous system.
The fact that so many cases of Guillain-Barr syndrome begin after a viral or bacterial
infection suggests that certain characteristics of some viruses and bacteria may activate
the immune system inappropriately. Investigators are searching for those
characteristics.